Unusual Sertoli Cell Tumor Associated With Sex Cord Tumor With Annular Tubules in Peutz-Jeghers Syndrome: Report of a Case and Review of the Literature on Ovarian Tumors in Peutz-Jeghers Syndrome

Int J Surg Pathol. 2016 May;24(3):269-73. doi: 10.1177/1066896915620663. Epub 2015 Nov 29.


We report the case of an 11-year-old girl with Peutz-Jeghers syndrome and a unilateral ovarian tumor most consistent with Sertoli cell tumor associated with sex cord tumor with annular tubules. The ovary was replaced by a lobular, solid, yellow tumor. Microscopic examination showed 2 components that focally merged. The first was composed of uniform, cytologically bland cells arranged mostly in diffuse sheets and focally in tubules. The second showed typical sex cord tumor with annular tubules with extensive calcification. The predominant component of the tumor clearly fell in the sex cord category and most closely resembled Sertoli cell tumor. This case adds to the limited information on ovarian sex cord tumors, other than typical sex cord tumor with annular tubules, arising in association with Peutz-Jeghers syndrome, a topic reviewed herein.

Keywords: Peutz-Jeghers syndrome; Sertoli cell tumor; ovarian neoplasms; sex cord stromal tumors.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Female
  • Humans
  • Neoplasms, Multiple Primary / genetics
  • Neoplasms, Multiple Primary / pathology
  • Ovarian Neoplasms / genetics
  • Ovarian Neoplasms / pathology*
  • Peutz-Jeghers Syndrome / complications*
  • Sertoli Cell Tumor / genetics
  • Sertoli Cell Tumor / pathology*
  • Sex Cord-Gonadal Stromal Tumors / genetics
  • Sex Cord-Gonadal Stromal Tumors / pathology*